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Under embargo until 11 a.m. ET Thursday, March 23
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Katelyn McCarthy
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Shape of inner ear helps predict hearing loss for children with rare
hearing
loss disorder
Findings could provide predictor for degree of hearing loss in children with enlarged vestibular
aqueduct
CLEVELAND, Ohio — It may be possible to predict the severity of hearing loss for children
diagnosed with enlarged vestibular aqueduct, according to a new study published in JAMAOtolaryngology-Head & Neck Surgery. This retrospective chart review, authored by physicians
and researchers within the University Hospitals Rainbow Babies & Children’s Enlarged
Vestibular Aqueduct (EVA) Research Project, is one of the first such studies to find a direct
connection between the increasing width of the vestibular aqueduct and the degree of hearing
loss a child experiences over time.
“There is still much to learn about the true causes and impacts of EVA,” says Mustafa Ascha,
MS, first author of the study and a researcher within the UH Rainbow Department of
Otolaryngology. “The hope is that these findings will help parents of young children with EVA,
and their physicians, better understand what lies ahead for their child’s hearing and speech based
on the severity of that child’s malformation.”
The study analyzed the medical records of 52 children diagnosed with EVA from the UH
Rainbow Department of Otolaryngology, selecting records with both a computerized
tomography (CT) scan for diagnosis and a median of five sets of audiogram test data to measure
speech reception threshold, or the minimum intensity of decibels at which a patient can
recognize half of spoken words, and the percent of words the patient recognized. The
longitudinal models used in the study are common in social sciences such as economics or
sociology, but applications in biomedical research are still gaining traction.
For each millimeter increase in vestibular aqueduct size greater than 1.5 millimeters, the research
team found an increase of 17.5 dB in speech reception threshold and a decrease of 21 percent in
word recognition scores. Additionally, for every year after the initial audiogram, the speech
recognition threshold for patients with EVA increased by 1.5 dB and word recognition decreased
by an additional 1.7 percent. They caution, though, that these results only represent averages for
patient hearing loss progression—EVA symptoms are highly variable.
Enlarged vestibular aqueduct is the most common inner ear malformation associated with
sensorineural hearing loss, which is caused by issues in the cochlea or the neural pathways that
carry sound to the brain. The malformation does not necessarily cause hearing loss, but both
EVA and hearing loss are caused by the same types of underlying defects. There is no treatment
specifically for EVA, however children experiencing hearing loss who have EVA are candidates
for hearing aids or cochlear implantation depending on the degree of hearing loss.
“Since EVA is considered an orphan disease, affecting fewer than 200,000 people per year,
focused research on it has been limited,” adds senior author Todd D. Otteson, MD, MPH, Chief
of Pediatric Otolaryngology at UH Rainbow. “This study may not change the treatment protocols
already in place for children diagnosed with EVA in the short term, but it will enable physicians
and families to appropriately plan a care trajectory that maximizes the child’s quality of life.”
The UH Rainbow Enlarged Vestibular Aqueduct Research Project is a donor-supported program
that aims to change the current research landscape for EVA syndrome by charting a new
pathway for clinical research discovery. The EVA Research Project is currently recruiting
participants for a national EVA patient registry to collect patient experiences. The voluntary
patient database will provide future researchers with the information necessary to better evaluate
and make recommendations for children with EVA.
About University Hospitals Rainbow Babies & Children’s Hospital
Internationally renowned, UH Rainbow Babies & Children’s Hospital is a full-service children’s hospital and
pediatric academic medical center with experts in 16 medical divisions and 11 surgical specialties who offer
nationally ranked care not available at other institutions in the region, including a center dedicated to adolescent and
young adult cancer treatment and Northeast Ohio’s only single-site provider of advanced maternal fetal medicine
and neonatology services. As an affiliate of Case Western Reserve University School of Medicine and the only
Level I Pediatric Trauma Center in the region, UH Rainbow Babies & Children’s Hospital offers access to novel
therapies, advanced technologies and clinical discoveries long before they are available nationwide. Rainbow
pediatric specialists – all of whom also serve on the faculty at the School of Medicine – are engaged in today’s most
advanced clinical research and are widely regarded as the best in the nation – and in some specialties, the best in the
world. Learn more at Rainbow.org.